|Year : 1967 | Volume
| Issue : 6 | Page : 241-242
Bilateral congenital anophthalmos
DL Maria, SK Srivastava, PS Sankholkar
Medical College, Aurangabad, India
|Date of Web Publication||22-Jan-2008|
D L Maria
Medical College, Aurangabad
Source of Support: None, Conflict of Interest: None
|How to cite this article:|
Maria D L, Srivastava S K, Sankholkar P S. Bilateral congenital anophthalmos. Indian J Ophthalmol 1967;15:241-2
Anophthalmos is a rare congenital anomaly. It is defined as a condition in which there is complete absence of one or both eyes. It is of great interest to embryologists, anatomists and ophthalmologists. The condition was first noted in 1557, but it was reliably reported by Bartholin in 1657 (SORSBY 1934). A few cases have been reported since (MANN and COLLINS 1887) Van HIPPEL 1900, WARFEL 1961, MICHAELS and ZUGSMITH 1963, IMAM 1964, MARIA and SHUKLA 1965, MEHRA 1965, INGLE, TALWALKAR and MARIA 1966. Because of the rarity of this anomaly and few reports from the country, this case of bilateral congenital anophthalmos is being reported.
| Case Report|| |
A two days old male child was referred to the ophthalmic Department from the Obstetric and Gynaecology department on 15-2-67 for examination of eyes. This child was the third son, the eldest one was male aged seven years, alive and the second was a female child, who died at two years of age. The last delivery was three years back. Both previous deliveries were full-term and normal. There was no congenital deformity.
| Examination|| |
The subject was a premature (32 weeks) male child weighing 1.75 Kg. Both the orbits were small, larger in the transverse diameter than the vertical. Both eye-lids and eye-lashes were present. The palpebral fissures were narrow but equal on both sides and the lacrimal puncta were present. The conjunctival sac was reduced in size.
The eye-ball could not be felt on palpation but movements of the conjunctival sac were noticed which must be due to the presence of extraocular muscles. The lids were kept closed. No other congenital abnormality was seen in the child. The orbits measured 25 mm in transverse diameter and 14 mm in vertical diameter, giving an orbital index of 56, normal value for the coloured being 84. The palpebral fissures measured 10 mm on both sides. The anterior and posterior fontanelle were normal. Other systems did not reveal any abnormality. Photos I and II.
| Discussion|| |
According to MANN (1937), there are three types of anophthalmos.
Primary Anophthalmos: There is a failure of formation of the optic pits which appear at 2 mm stage. The defect is restricted to the optic pits only, since the rest of the brain develops normally. Usually, the condition is bilateral and sporadic. The orbit and the other mesodermal structures develop normally though they may be malformed.
Secondary Anophthalmos: This is due to absence or abnormal development of the fore-brain as a whole. It usually results in nonviable monsters.
Degenerative anophthalmos: This is due to the subsequent degeneration of a primarily formed optic vesicle. The etiology is suggested by the presence of a small fibrous nodule or rudimentary lens structure. There is usually no gross anomaly of the brain. There does not appear to be any particular sex incidence, nor is there any evidence of parental transmission except in rare cases. van DUNSE (1899) postulated an inconvincing theory of suppression of optic vesicle by thickened amnion.
Germinal influence is present in cases associated with other congenital anomalies. Genetic and chromosomal abnormalities may be responsible in few cases. The sporadic and isolated occurrence of anophthalmos with no history of disease or consanguinity suggests an environmental cause. This is supported by experimental work wherein suppression of the optic vesicles in animals has been produced by teratogens like X-rays, deficiency or excess of vitamin A etc. It may be concluded that there is no one cause but multiple factors responsible in the etiology of anophthalmos.
| Summary|| |
A case of bilateral anophthalmos of the primary type in a third male offspring of a family has been reported. The subject is reviewed in brief.
| References|| |
BARTHOLIN (1657) Hist. anatomicarum rariorum Amsterdam quoted in 3.
COLLINS (1887) Roy Lond. Ophthal. Hosp. Rep. 11, 429 quoted in 3.
DUKE ELDER, S. (1964) System of Ophthalmology Vol. III Part 2 Henry Kimpton, London P. 416.
INGLE V. N., TALWALKAR B., MARIA D. L. 1966 - Ind. J. Pediat, 33; 149.
IMAM S. N. (1964) Patna J. of Med. ophthalmic special 242-243.
MANN IDA (1937) "Developmental Abnormalities of Eye" Cambridge P. 65-72.
MARIA D. L. and SHUKLA S. P. (1965) J. All India opthalmic Soc. 13, 121.
MEHRA, K. S. (1965) Anophthalmos amer. J. Ophthal. 60, 341.
MICHAELS D. D. and ZUGSMITH G. S. (1963) Amer. J. Ophthal. 50, 1256.
SORSBY. A. (1934) Brit. J. Ophtha. 18, 469.
VAN DUYSE (1899) Arch. Ophthal. (Paris) 19412.
WARFEL J. J. (1961) Amer. J. Ophthal. 51, 698.
[Figure - 1], [Figure - 2]